A clinico-pathological study of a case of kuru
Abstract
Kuru was diagnosed in a 42-year-old Melanesian male from the Eastern Highlands Province of Papua New Guinea. The clinical features indicated predominant cerebellar degeneration together with widespread cortical neuronal dysfunction and involvement of the diencephalon, hippocampus and basal ganglia. Dementia was an early and prominent feature. The duration of clinical illness was about 12 months and atypically he spent the last 7 months in hospital allowing continuous assessment. At autopsy, spongiform encephalopathy was demonstrated, and inoculation of brain tissue into 4 squirrel monkeys and 1 capuchin monkey resulted in the development of kuru. This is the longest continuous study of kuru in a hospital setting to be recorded in the adult human subject. The virus isolated from the brain of this patient has been adopted as a standard reference strain of kuru for future use and repository.
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The most striking neuropathologic feature of kuru is the presence of numerous amyloid plaques, known as kuru plaques.
“The most striking neuropathologic feature of kuru is the presence of numerous amyloid plaques described as “spherical bodies with a rim of radiating filaments” and found in 6 of 12 cases studied by Klatzo et al (62) and in “about three quarters” of the 13 cases of Beck and Daniel (68); they then became known as “kuru plaques” (67, 69–71).”
From Kuru (2012) · cites this paper as [70]
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References 14 · Cited by 37
- Authors
- Scrimgeour, E. M., Masters, C. L., Alpers, M. P., Kaven, J., & Gajusek, D. C.
- Affiliations
- University of Papua New Guinea (PG)
- National Institutes of Health (US)
- Papua New Guinea Institute of Medical Research (PG)
- ORCID
- Masters, Colin L.
- Venue
- Journal of the Neurological Sciences
- Publisher
- Elsevier BV
- Published
- 1983-05
- Volume
- 59
- Issue
- 2
- Pages
- 265-275
- ISSN
- 0022-510X (ISSN-L)
- OpenAlex
- W2037719793
- Cited by
- 37
- References
- 14
- Citations by year
- 2024: 1 · 2023: 19 · 2021: 1 · 2019: 1 · 2017: 1 · 2013: 2 · 2012: 1
- FWCI
- 0.15
- Type
- journal article
- Language
- en
- Open access
- closed
- Locations
- Journal of the Neurological Sciences (publishedVersion)
- PubMed (publishedVersion)
- Topics
- Life Sciences › Biochemistry, Genetics and Molecular Biology › Molecular Biology › Prion Diseases and Protein Misfolding
- Life Sciences › Biochemistry, Genetics and Molecular Biology › Molecular Biology › Amyloidosis: Diagnosis, Treatment, Outcomes
- Life Sciences › Neuroscience › Cellular and Molecular Neuroscience › Genetic Neurodegenerative Diseases
- Keywords
- kuru, spongiform encephalopathy, prion disease, cerebellar degeneration, dementia, diencephalon, hippocampus, basal ganglia, clinical course, neuropathology, reference strains, Melanesians
- MeSH terms
- Adult, Autopsy, Brain, Humans, Kuru, Male, Pyramidal Tracts
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